Idiopathic pulmonary fibrosis is a disease in which lung tissue hardens and is an incurable disease of unknown cause. Generally, it occurs most often between the ages of 50 and 80, and it is known that men are at higher risk than women.
Dry cough and shortness of breath are the main symptoms.
Idiopathic pulmonary fibrosis is a disease that causes chronic inflammatory cells to infiltrate into the walls of the alveoli and causes various changes that harden the lungs, leading to a gradual decline in lung function, leading to death.
The prognosis is so poor that more than 50% of patients diagnosed with idiopathic pulmonary fibrosis will die within 3 to 5 years. It is known that once the disease progresses and the lung tissue is completely hardened with fibrosis, no matter what treatment, it does not improve.
The cause of idiopathic pulmonary fibrosis is still unknown. The most widely accepted hypothesis is that the disease is caused by the constant application of unknown stimuli to susceptible individuals. The incidence is high in smokers, and risk factors such as antidepressants, chronic lung inhalation due to gastroesophageal reflux, and inhalation of metal dust or wood dust are reported as risk factors.
The typical symptoms of idiopathic pulmonary fibrosis are dry cough that progresses slowly over 1 to 2 years and dyspnea during exercise. Experts say that if the cough does not go away or if breathing difficulties persist, it may be idiopathic pulmonary fibrosis.
In the case of idiopathic pulmonary fibrosis, as the disease progresses, breathing difficulties become more severe, making daily life difficult later on. When hypertension is accompanied by high blood pressure in patients with end-stage idiopathic pulmonary fibrosis, the heart is burdened.
Consider lung transplantation if medication is not effective
There is no effective treatment for idiopathic pulmonary fibrosis. It is for this reason that not all patients are treated even if they are diagnosed with idiopathic pulmonary fibrosis in a hospital. In general, it is recommended to treat patients diagnosed with idiopathic pulmonary fibrosis if there is a pulmonary dysfunction in clinical or pulmonary function tests, or if lung function declines during follow-up.
The standard treatment for idiopathic pulmonary fibrosis is medication. The antifibrotic drugs pirfenidone and nintedanib have been shown to slow disease progression and are considered standard treatments. Steroids are sometimes used when lung function deteriorates acutely.
Lung transplantation may be considered if it becomes more severe and life-threatening despite medication. Lung transplantation can be attempted if you do not have a specific disease and are not elderly. The survival period following lung transplantation varies, but is generally confirmed to be 6-7 years.